Vasculitis



Last Review Date: December 26, 2016


What is vasculitis?

Vasculitis refers to a rare group of conditions characterised by inflammation of blood vessels. It occurs when the immune system attacks a person's own blood vessels. The cause of vasculitis is often unknown. In the absence of an identifiable cause, it is called primary vasculitis. When an underlying cause can be identified, it is called secondary vasculitis. Secondary vasculitis may be triggered by an allergic reaction to a medication, in response to an infection such as hepatitis C, or as a result of an autoimmune disease, such as rheumatoid arthritis. In some cases, secondary vasculitis is seen as a complication of blood cell cancers such as leukaemia and lymphoma.

Vasculitis can affect any type of blood vessel, including veins, arteries, and capillaries. It can result in narrowed or blocked vessels that limit the circulation of blood, leading to tissue or organ damage. In some cases, a bulge in a weakened vessel, called an aneurysm, can occur. An aneurysm can be a life-threatening complication because it can rupture, leading to heavy internal bleeding.

There are many types of vasculitis, which are often classified according to the size of the affected blood vessels. On the next page is a table containing examples of types of vasculitis.


Types and Symptoms of Vasculitis

Because vasculitis is a systemic illness, symptoms can be non-specific and include those typical of inflammation, such as:

  • Fatigue
  • Loss of appetite
  • Weight loss
  • Fever
  • Body aches

These symptoms may appear rapidly or may develop gradually over the course of several months. The presentation of symptoms can vary considerably from person to person. In addition to these diffuse symptoms, someone can have organ-specific, localised symptoms associated with the particular type of vasculitis they have. The size and specific blood vessels that are affected often lead to a characteristic set of symptoms for each type. The table below provides examples of some types of vasculitis.

Name

Blood Vessels Affected

Description and Symptoms

Behçet's Disease

Large, medium, and small

Characterized by painful mouth and genital sores and eye inflammation but can affect other organs and tissues as well, including the skin, lungs, and joints; tends to affect men more than women

Giant Cell Arteritis (GCA)

Large

Also called temporal arteritis because it involves inflammation of the temporal arteries along the sides of the head near the temples; associated with headaches, scalp tenderness, jaw pain, and blurred vision; most common type of vasculitis; most often affects women and people over age 50

Polymyalgia Rheumatica

Large

Occurs in half of those with GCA (above); characterised by pain and stiffness in the shoulders and hips; found mostly in those over age 50

Takayasu's Arteritis

Large, medium

Affects the largest blood vessel in the body – the aorta – and its branches; most cases occur in young women, especially Asian women, and it can start in childhood; general symptoms of malaise, fatigue, fever, arm weakness, joint pain, headaches, and loss of appetite

Buerger's Disease

Medium

Characterized by reduced blood flow to the hands and feet, resulting in numbness and tingling, and can lead to gangrene; associated with cigarette smoking

Central Nervous System Vasculitis

Medium

Very rare type of vasculitis in which involvement is confined to the central nervous system (brain and spinal cord); symptoms include headache, changes in mental function, and muscle weakness or paralysis

Kawasaki Disease

Mostly medium, but can affect any blood vessel

Rare form of vasculitis that affects children, especially those under age 5, and causes a fever that does not respond to medication and redness of the mucous membranes (eyes, mouth) and skin rash as well as enlarged lymph nodes

Polyarteritis Nodosa

Medium

Affects the skin, heart, kidneys, and central nervous system; associated with fever, weight loss, muscle and joint pain as well as anaemia

Churg-Strauss Syndrome - 
now termed eGPA (eosinophilic granulomatosis with polyangiitis)

Small

Characterised by inflammation of the nose, asthma, and skin and lung involvement, but can also affect other organ systems; very high number of eosinophils (type of white blood cell) may be found in blood and tissues; associated with granulomas (lumps due to tissue inflammation)

Henoch-Schönlein Purpura

Small

Most common type of vasculitis in children; usually follows an upper respiratory tract infection and characterised by inflammation of blood vessels in the skin, causing purplish spots or patches (purpura) that may present on the arms and legs; also associated with abdominal pain and blood in the urine; usually resolves on its own within a few weeks

Essential Mixed Cryoglobulinaemia

Small

Often associated with chronic hepatitis C infection and occurs as an immune response to that infection; characterised by purpura usually on the lower extremities but also can cause joint pain, weakness, limb tingling/numbness, and kidney inflammation

Microscopic Polyangiitis

Small, medium

Affects vessels in various organs, including the kidneys, lungs, nervous system, and skin; general symptoms include fever, muscle aches, and weight loss while organ-specific symptoms can include coughing up blood

Granulomatosis with polyangiitis (previously called Wegener's Granulomatosis)

Small

Rare form of vasculitis that can involve the upper respiratory tract, lungs, kidneys, and other organs; granulomas can occur such as in the lungs and cause damage; general symptoms include fever, muscle aches, and weight loss while more specific symptoms can include shortness of breath and chronic sinusitis and ear infections

For more information on each of these conditions, see the web sites of the Vasculitis Foundation and the Johns Hopkins Vasculitis Center. For more information on symptoms by type of vasculitis, see the National Heart Lung and Blood Institute: Types of Vasculitis.


Testing

A tissue biopsy is the gold standard test for diagnosis of vasculitis. It involves taking a tissue sample from a blood vessel or affected organ and examining it for signs of inflammation or damage. This is the only way to be sure of a diagnosis of vasculitis; however, it is invasive, so a combination of laboratory and non-laboratory tests, in addition to a review of signs and symptoms, medical history, and a physical exam, can be used to help in decision-making. For example, tests can help pinpoint an organ involved on which to perform a biopsy to confirm or exclude a diagnosis of vasculitis. Several of these tests are also used to monitor treatment.

Laboratory tests
  • Full Blood Count (FBC) – this test is used to look for complications of vasculitis and its treatment. It evaluates the patient's red blood cells and haemoglobin for anaemia and checks the white blood cell count, which can be increased in infection and reduced with some treatments. Higher than normal numbers of white blood cells are seen with some types of vasculitis, such as Churg-Strauss Syndrome.
  • Erythrocyte sedimentation rate (ESR) – this test shows the presence of inflammation in the body and can be increased with some types of vasculitis, such as microscopic polyangitis and Granulomatosis with polyangiitis (previously known as Wegener's Granulomatosis.)
  • C-reactive protein test (CRP) - this test also detects inflammation in the body.
  • Creatinine – this test assesses kidney function to determine if the vasculitis is affecting the kidneys.
  • Liver panel – these tests assess liver function to determine if the vasculitis is affecting the liver.
  • Urinalysis – this test looks for presence of protein and red blood cells in the urine, which can indicate inflammation in the kidney as can occur with some types of vasculitis.
  • Anti-neutrophil cytoplasmic antibody (ANCA) – this test is a useful marker for primary systemic vasculitis conditions, such as Granulomatosis with polyangiitis (previously known as Wegener's Granulomatosis) and Microscopic Polyangitis.
  • Complement – part of the inflammatory response often reduced with vasculitis when immune complex-related conditions such as this are present.
Non-laboratory tests
  • Radiological imaging – chest x-ray, EKG, echocardiography, CT scan, MRI can be used to help determine if large arteries are affected.
  • Lung function tests – to determine if airflow is restricted when vasculitis is affecting the lungs.
  • Nerve conduction studies or nerve biopsy may be useful if there are symptoms of numbness or tingling.
  • Angiography, an x-ray examination of the blood vessels after dye has been injected into the bloodstream, can be useful in some types of vasculitis.

For more on imaging studies, see the web sites Inside Radiology (Australian) and Radiologyinfo.org (US).


Treatment

The treatment of vasculitis depends primarily on whether it is primary or secondary. If an underlying cause can be identified (secondary vasculitis), treatment should be directed at the cause. Examples include withdrawal of a triggering drug or treatment of an underlying infection.

Since vasculitis results from an "overactive" immune system, treatment often involves drugs that suppress the immune system. The most commonly used drugs are corticosteroids, such as prednisone and methylprednisolone. Unfortunately, when used long term, they are associated with side effects such as weight gain, reduced bone density, and increased risk of diabetes and high blood pressure. In order to reduce the corticosteroid requirements and still control the disease, other immunosuppressive drugs such as cyclophosphamide, methotrexate, and azathioprine may be used.

The choice of drug for each patient should be decided after considering the severity of the disease. All immunosuppressive drugs carry an increased risk of infection and potential for reduction in blood cell counts. Frequent monitoring, using blood tests such as full blood cell counts (FBC)liver function tests, and kidney function tests (creatinineureaurinalysis), helps to detect such side effects early. The use of other medications, such as folic acid in patients taking methotrexate, can protect the individual from some of the side effects.


Related Pages

On This Site
Tests: ANCA/MPO/PR3 AntibodiesComplement
Conditions: Autoimmune DisordersHypercoagulable Disorders

Elsewhere On The Web
healthdirect Australia: Vasculitis
The Johns Hopkins Vasculitis Center (USA)
Vasculitis Foundation (USA)
MedlinePlus: Vasculitis (USA)
National Heart Lung and Blood Institute: Vasculitis (USA)
Mayo Clinic: Vasculitis (USA)